CARDIAC IRON OVERLOAD IN THALASSEMIC PATIENTS - AN ENDOMYOCARDIAL BIOPSY STUDY
CARDIAC IRON OVERLOAD IN THALASSEMIC PATIENTS - AN ENDOMYOCARDIAL BIOPSY STUDY
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DOI：
10.1007/bf01702649
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发表时间：
1995-09-01
影响因子：
3.5
通讯作者：
CALVI, V
中科院分区：
文献类型：
--
作者：
LOMBARDO, T;MORRONE, ML;CALVI, V
由器官铁质沉着症引起的继发性心力衰竭是重型地中海贫血患者死亡的主要原因。目前尚不能预测心脏铁质沉着症是否与铁超载有关，对重型地中海贫血和中间型地中海贫血患者输血后血色沉着症的真实的心脏组织学模式知之甚少。本研究旨在通过无创和有创技术评价心脏铁负荷。对15例地中海贫血患者进行了研究，其中10例进行了肌内膜活检，发现不同程度的肌内膜铁过载，组织化学阳性。非侵入性技术不能提供心脏血色病的确切图像。血清铁蛋白水平与心肌铁分级有显著相关性，铁蛋白水平升高和对螯合治疗依从性差的患者是严重心脏血色病的高危人群，肌内膜活检是研究心肌铁的有用工具。
Secondary heart failure induced by organ siderosis is the main cause of death in patients affected by thalassemia major. At present it cannot be predicted whether heart siderosis is correlated with iron overload and little is known about the real cardiac histological pattern of post transfusional hemochromatosis in patients with thalassemia major and intermedia. The study aim was to evaluate cardiac iron overload by non invasive and invasive techniques. Fifteen thalassemic patients were investigated and endomyocardial biopsy performed in ten revealed different grades of endomyocardial iron overload with histochemical positivity. Non invasive techniques are not able to furnish an exact picture of the cardiac hemochromatosis. There was a significant correlation between serum ferritin and myocardial iron grade.Patients with elevated ferritin levels and poor compliance to chelating therapy are at high risk of severe heart hemochromatosis, It was seen that endomyocardial biopsy is a useful tool in studying myocardial iron.